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B-type lamins in health and disease (2014)
Journal Article
Hutchison, C. (2014). B-type lamins in health and disease. Seminars in Cell and Developmental Biology, 29, 158-163. https://doi.org/10.1016/j.semcdb.2013.12.012

For over two decades, B-type lamins were thought to have roles in fundamental processes including correct assembly of nuclear envelopes, DNA replication, transcription and cell survival. Recent studies have questioned these roles and have instead emp... Read More about B-type lamins in health and disease.

The accumulation of un-repairable DNA damage in laminopathy progeria fibroblasts is caused by ROS generation and is prevented by treatment with N-acetyl cysteine (2011)
Journal Article
Richards, S., Muter, J., Ritchie, P., Lattanzi, G., & Hutchison, C. (2011). The accumulation of un-repairable DNA damage in laminopathy progeria fibroblasts is caused by ROS generation and is prevented by treatment with N-acetyl cysteine. Human Molecular Genetics, 20(20), 3997-4004. https://doi.org/10.1093/hmg/ddr327

Fibroblasts from patients with the severe laminopathy diseases, Restrictive Dermopathy (RD) & Hutchinson Gilford Progeria Syndrome (HGPS), are characterised by poor growth in culture, the presence of abnormally shaped nuclei and the accumulation of D... Read More about The accumulation of un-repairable DNA damage in laminopathy progeria fibroblasts is caused by ROS generation and is prevented by treatment with N-acetyl cysteine.

Conserved cysteine residues in the mammalian lamin A tail are essential for cellular responses to ROS generation (2011)
Journal Article
Pekovic, V., Gibbs-Seymour, I., Markiewicz, E., Alzoghaibi, F., Benham, A., Edwards, R., …Hutchison, C. (2011). Conserved cysteine residues in the mammalian lamin A tail are essential for cellular responses to ROS generation. Aging Cell, 10(6), 1067-1079. https://doi.org/10.1111/j.1474-9726.2011.00750.x

Pre-lamin A and progerin have been implicated in normal aging, and the pathogenesis of age-related degenerative diseases is termed ‘laminopathies’. Here, we show that mature lamin A has an essential role in cellular fitness and that oxidative damage... Read More about Conserved cysteine residues in the mammalian lamin A tail are essential for cellular responses to ROS generation.

A Flow Cytometry-Based Screen of Nuclear Envelope Transmembrane Proteins Identifies NET4/Tmem53 as Involved in Stress-Dependent Cell Cycle Withdrawal (2011)
Journal Article
Korfali, N., Srsen, V., Waterfall, M., Batrakou, D. G., Pekovic, V., Hutchison, C. J., & Schirmer, E. C. (2011). A Flow Cytometry-Based Screen of Nuclear Envelope Transmembrane Proteins Identifies NET4/Tmem53 as Involved in Stress-Dependent Cell Cycle Withdrawal. PLoS ONE, 6(4), Article e18762. https://doi.org/10.1371/journal.pone.0018762

Disruption of cell cycle regulation is one mechanism proposed for how nuclear envelope protein mutation can cause disease. Thus far only a few nuclear envelope proteins have been tested/found to affect cell cycle progression: to identify others, 39 n... Read More about A Flow Cytometry-Based Screen of Nuclear Envelope Transmembrane Proteins Identifies NET4/Tmem53 as Involved in Stress-Dependent Cell Cycle Withdrawal.

Lamin A/C is a risk biomarker in colorectal cancer (2008)
Journal Article
Willis, N., Cox, T., Rahman-Casans, F., Smit, K., Przyborski, S., van den Brandt, P., …Hutchison, C. (2008). Lamin A/C is a risk biomarker in colorectal cancer. PLoS ONE, 3(8), Article e2988. https://doi.org/10.1371/journal.pone.0002988

Background: A-type lamins are type V intermediate filament proteins encoded by the gene LMNA. Mutations in LMNA give rise to diverse degenerative diseases related to premature ageing. A-type lamins also influence the activity of the Retinoblastoma pr... Read More about Lamin A/C is a risk biomarker in colorectal cancer.

A novel role for the nuclear membrane protein emerin in association of the centrosome to the outer nuclear membrane (2007)
Journal Article
Salpingidou, G., Smertenko, A., Hausmanowa-Petrucewicz, I., Hussey, P., & Hutchison, C. (2007). A novel role for the nuclear membrane protein emerin in association of the centrosome to the outer nuclear membrane. Journal of Cell Biology, 178(6), 897-904. https://doi.org/10.1083/jcb.200702026

The type II inner nuclear membrane protein emerin is a component of the LINC complex that connects the nuclear lamina to the actin cytoskeleton. In emerin-null or -deficient human dermal fibroblasts we find that the centrosome is detached from the nu... Read More about A novel role for the nuclear membrane protein emerin in association of the centrosome to the outer nuclear membrane.

Nucleoplasmic LAP2 alpha-lamin A complexes are required to maintain a proliferative state in human fibroblasts (2007)
Journal Article
Pekovic, V., Harborth, J., Broers, J., Ramaekers, F., van Engelen, B., Lammens, M., …Markiewicz, E. (2007). Nucleoplasmic LAP2 alpha-lamin A complexes are required to maintain a proliferative state in human fibroblasts. Journal of Cell Biology, 176(2), 163-172. https://doi.org/10.1083/jcb.200606139

In human diploid fibroblasts (HDFs), expression of lamina-associated polypeptide 2 (LAP2) upon entry and exit from G0 is tightly correlated with phosphorylation and subnuclear localization of retinoblastoma protein (Rb). Phosphoisoforms of Rb and LAP... Read More about Nucleoplasmic LAP2 alpha-lamin A complexes are required to maintain a proliferative state in human fibroblasts.

The inner nuclear membrane protein Emerin regulates beta-catenin activity by restricting its accumulation in the nucleus (2006)
Journal Article
Markiewicz, E., Tilgner, K., Barker, N., van de Wetering, M., Clevers, H., Dorobek, M., …Hutchison, C. (2006). The inner nuclear membrane protein Emerin regulates beta-catenin activity by restricting its accumulation in the nucleus. The EMBO Journal, 25(14), 3275-3285. https://doi.org/10.1038/sj.emboj.7601230

Emerin is a type II inner nuclear membrane (INM) protein of unknown function. Emerin function is likely to be important because, when it is mutated, emerin promotes both skeletal muscle and heart defects. Here we show that one function of Emerin is t... Read More about The inner nuclear membrane protein Emerin regulates beta-catenin activity by restricting its accumulation in the nucleus.

Lamina-associated polypeptide 2 alpha regulates cell cycle progression and differentiation via the retinoblastoma-E2F pathway (2006)
Journal Article
Dorner, D., Vlcek, S., Foeger, N., Gajewski, A., Makolm, C., Gotzmann, J., …Foisner, R. (2006). Lamina-associated polypeptide 2 alpha regulates cell cycle progression and differentiation via the retinoblastoma-E2F pathway. Journal of Cell Biology, 173(1), 83-93. https://doi.org/10.1083/jcb.200511149

Lamina-associated polypeptide (LAP) 2 is a nonmembrane-bound LAP2 isoform that forms complexes with nucleoplasmic A-type lamins. In this study, we show that the overexpression of LAP2 in fibroblasts reduced proliferation and delayed entry into the ce... Read More about Lamina-associated polypeptide 2 alpha regulates cell cycle progression and differentiation via the retinoblastoma-E2F pathway.

Lamin A/C binding protein LAP2 alpha is required for nuclear anchorageof retinoblastoma protein (2002)
Journal Article
Markiewicz, E., Dechat, T., Foisner, R., Quinlan, R., & Hutchison, C. (2002). Lamin A/C binding protein LAP2 alpha is required for nuclear anchorageof retinoblastoma protein. Molecular Biology of the Cell, 13(12), 4401-4413. https://doi.org/10.1091/mbc.e02-07-0450

The phosphorylation-dependent anchorage of retinoblastoma protein Rb in the nucleus is essential for its function. We show that its pocket C domain is both necessary and sufficient for nuclear anchorage by transiently expressing green fluorescent pro... Read More about Lamin A/C binding protein LAP2 alpha is required for nuclear anchorageof retinoblastoma protein.